You are in: eMedicine Specialties > Ophthalmology > RETINA Terson SyndromeArticle Last Updated: Jul 18, 2007AUTHOR AND EDITOR INFORMATIONSection 1 of 12
  Author: Richard J Ou, MD, Clinical Assistant Professor, Baylor College of Medicine; Attending Physician and Consultant, Michael E DeBakey Veterans Affairs Medical Center Richard J Ou is a member of the following medical societies: American Academy of Ophthalmology, American Medical Association, Harris County Medical Society, and Texas Medical Association Coauthor(s): Marc O Yoshizumi, MD, Director of Eye Trauma and Emergency Center, Professor, Department of Ophthalmology, Jules Stein Eye Institute, University of California at Los Angeles Editors: Brian A Phillpotts, MD, Former Vitreo-Retinal Service Director, Former Program Director, Clinical Assistant Professor, Department of Ophthalmology, Howard University College of Medicine; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine; Steve Charles, MD, Director of Charles Retina Institute; Clinical Professor, Department of Ophthalmology, University of Tennessee College of Medicine; Lance L Brown, OD, MD, Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri; Hampton Roy Sr, MD, Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences Author and Editor Disclosure Synonyms and related keywords: vitreous hemorrhage, subhyaloid hemorrhage, retinal hemorrhage, subarachnoid hemorrhage, intraocular hemorrhage, intracranial bleeding, increased intracranial pressure INTRODUCTIONSection 2 of 12
  In 1881, Litten first described an intraretinal hemorrhage associated with subarachnoid hemorrhage in the German literature. However, Terson's description of vitreous hemorrhage following subarachnoid hemorrhage in 1900 is now associated with this syndrome. ProblemTerson syndrome originally was defined by the occurrence of vitreous hemorrhage in association with subarachnoid hemorrhage. Terson syndrome now encompasses any intraocular hemorrhage associated with intracranial hemorrhage and elevated intracranial pressures. Intraocular hemorrhage includes the development of subretinal, retinal, preretinal, subhyaloidal, or vitreal blood. The classic presentation is in the subhyaloidal space. FrequencyReports have shown an incidence of 10-50% of intraocular hemorrhage with subarachnoid hemorrhage. This association is statistically associated with the severity of the subarachnoid hemorrhage based on the Hunt-Hess classification system of subarachnoid hemorrhages. The incidence of vitreous hemorrhage is much lower (3-13%). Papilledema and unconsciousness are both positively correlated with Terson syndrome. EtiologyTerson syndrome has been described most commonly in subarachnoid hemorrhages due to ruptured cerebral aneurysms. Although early studies attempted to link this syndrome with aneurysms of the anterior communicating artery, statistical analysis has not correlated it with a specific aneurysmal location. Other reports include such causes as strangulation, trauma, tumor, and postsurgical intracranial bleeding. PathophysiologyThe pathogenesis of Terson syndrome has been controversial. The earliest reports assumed that the intracerebral blood directly connected with the intraocular space through the lamina cribrosa. Electron microscopy of the optic nerve anatomy has not demonstrated a communication between the 2 spaces. In addition, pathological specimens have not shown any blood in the optic nerve sheath within 3 mm of the globe. Another mechanism suggests that a sudden rise in the venous pressure caused by the intracerebral bleeding is transmitted to the eye and results in intraocular bleeding. However, experimental studies have shown that the intravenous pressures are not high enough to create an intraocular hemorrhage. The sudden rise in intracranial pressure is probably the primary inciting event in Terson syndrome. Intracranial pressure is transmitted through the optic nerve sheath to the swollen optic nerve head, which occludes the retinal and choroidal anastomoses at the level of the lamina cribrosa. The elevated venous pressure generated in the retinal venous system is assumed to rupture the superficial retinal vessels, resulting in intraocular hemorrhages. A recent case report supports this theory by demonstrating peripapillary fluorescein leakage in a patient with vitreous hemorrhage secondary to a subarachnoid hemorrhage. Because of these assumptions regarding the pathogenesis of the syndrome, the definition of Terson syndrome now includes any intraocular hemorrhage associated with intracranial bleeding and acutely increased intracranial pressure. Attempts have been made to correlate Terson syndrome with the intraocular hemorrhages seen in shaken baby syndrome because of the similarity in clinical findings in the eye and the brain, but acute tractional forces may be an additional factor contributing to the intraocular hemorrhage in the latter. ClinicalThe neurologic symptoms are related to intracranial bleeding. Reported visual acuities range from 20/20 to light perception, but they often are difficult to obtain secondary to the impaired neurologic status of the patient. The degree of visual loss is related to the degree and extent of the intraocular hemorrhage. The intraocular hemorrhage is usually bilateral and superficial to the retina. Intraretinal or subretinal hemorrhages have been reported but are less frequent. Preretinal hemorrhage can develop into vitreous hemorrhage weeks after the initial inciting event. The intraocular hemorrhage may be difficult to diagnose immediately because the ophthalmologist is restricted from dilating the patient for neurologic monitoring. A decreased red reflex is helpful in evaluating a patient who is comatose, and B-scan ultrasound can further establish the extent of vitreous hemorrhage. INDICATIONSSection 3 of 12
Strict guidelines for treatment have not been established by clinical studies. Patients usually have responded well to observation.
RELEVANT ANATOMYSection 4 of 12
The relevant anatomy in Terson syndrome includes the inner retinal vasculature, choroidal vasculature, chorioretinal anastomoses near the optic nerve head, and subarachnoid space surrounding the optic nerve. CONTRAINDICATIONSSection 5 of 12
Contraindications for a vitrectomy include a small intraocular hemorrhage with a high likelihood of spontaneous clearing and an intraocular hemorrhage that is spontaneously clearing. WORKUPSection 6 of 12
Lab Studies
Imaging Studies
Other Tests
Diagnostic Procedures
Histologic FindingsPathologic specimens of patients with Terson syndrome have shown abundant erythrocytes with occasional leukocytes in the vitreous, subhyaloidal and subinternal limiting membrane space, and retina. Clinical case reports have documented subretinal blood, but this is not as common. Epiretinal membranes examined in Terson syndrome show glial cells and basement membrane material. StagingNo staging currently exists. TREATMENTSection 7 of 12
Medical therapySpontaneously clearing vitreous hemorrhage or small intraocular hemorrhage is most common. Elevated head positioning with bed rest and avoidance of anticoagulation medications (eg, aspirin, nonsteroidal anti-inflammatory drugs [NSAIDs], warfarin) may be helpful. Resolution of symptoms may take months. One study demonstrated an average of 9 months for the clearance of such hemorrhages. Surgical therapyA large vitreous hemorrhage may not clear spontaneously and may require a vitrectomy to facilitate clearing. In pediatric cases, the development of amblyopia is a consideration for early vitrectomy. A vitrectomy with possible epiretinal membrane peeling may be necessary for late complications (eg, macular pucker). Preoperative detailsPerform a B-scan ultrasound examination. Consult with a neurologist to approve of the patient's neurologic stability for surgery. Intraoperative detailsIntervention with a vitrectomy is rarely needed in most patients with Terson syndrome because of the spontaneous clearing of both the vitreous and the preretinal or subhyaloidal hemorrhage. Special consideration should be made for early intervention in those patients where amblyopia is concerned. For long-standing vitreous hemorrhages of over 4- to 6-month duration without signs of progressive clearing, a core vitrectomy can be performed using the standard 3-port vitrectomy technique. In patients with loculated, nonclearing preretinal or subhyaloidal hemorrhage, the posterior hyaloid face can be incised and stripped away from the retinal surface with a bent microvitreoretinal (MVR) blade following a core vitrectomy. The use of YAG laser photodisruption of the preretinal or posterior hyaloid membrane has been used for the treatment of subhyaloidal hemorrhages due to other mechanisms, but it is not recommended for patients with Terson syndrome because of the potential for the development of late-stage macular pucker or epiretinal membrane formation. This late complication of Terson syndrome is best addressed by posterior hyaloid membrane stripping using a bent MVR blade and excision of the peeled membrane with the vitrectomy instrument. Postoperative detailsAvoid anticoagulation therapy. Follow-upCoordinate postoperative care with a neurologist. COMPLICATIONSSection 8 of 12
The most common complication is the formation of epiretinal membranes, observed in 27-78% of patients with Terson syndrome. The subhyaloidal or subinternal limiting membrane space created by the intraocular hemorrhage may result in fibroblast or glial cell proliferation. The resulting macular pucker or epiretinal membrane formation may severely affect vision after the resolution of the hemorrhage and may become visually significant as late as 4 years after the resolution of the hemorrhage. Other reported long-term complications include retinal pigment epithelium mottling, optic atrophy, macular holes, retinal folds, cystoid retinal changes, proliferative vitreoretinopathy, retinal detachment, and cataract formation. OUTCOME AND PROGNOSISSection 9 of 12
Terson syndrome has been correlated significantly in several studies with elevated morbidity and mortality when compared with subarachnoid hemorrhage without intraocular bleeding. Vitreous hemorrhage is associated with a 3- to 9-fold higher rate of mortality in comparison to other sites of intraocular bleeding in Terson syndrome. Patients able to survive the neurologic complications usually have a favorable visual prognosis. In a study of 30 patients with Terson syndrome, over 83% of patients achieved a long-term visual acuity better than 20/50 following observation or a vitrectomy. No statistical difference in final visual acuity was found between patients treated with observation or a vitrectomy. The most common cause of persistent visual loss is epiretinal membrane formation as a late complication of Terson syndrome. A nonclearing vitreous hemorrhage in an infant may lead to amblyopia and warrants early vitrectomy. FUTURE AND CONTROVERSIESSection 10 of 12
Observation usually results in a favorable visual outcome for patients with Terson syndrome. Ophthalmic surgical intervention may be necessary to treat late complications (eg, macular epiretinal membranes). Immediate vitrectomy for intraocular hemorrhages is not recommended, except for patients with submacular hemorrhage, patients who are monocular with severe visual loss, or pediatric patients at risk for amblyopia. The exact etiology of the mechanism of the intraocular blood remains to be elucidated definitively through histologic or experimental models. MULTIMEDIASection 11 of 12
REFERENCESSection 12 of 12
Article Last Updated: Jul 18, 2007 | |||||||||||||||||||
